Variability of response to early puberty induction demonstrated by transverse uterine diameter measurement and a novel method of 3D breast imaging.

OBJECTIVE: Induction of puberty with exogenous oestrogen results in considerable variability in final uterine and breast volumes. We set out to quantify the variability of these two outcome measures with a view to establishing monitoring methods that could be used to individualise treatment protocols. DESIGN: A prospective observational study. PARTICIPANTS: Sixteen participants with pubertal delay and primary amenorrhoea, due to hypogonadism were recruited from paediatric gynaecology and endocrinology clinics at University College London Hospital. A standardised protocol of transdermal 17ß oestradiol (17ßE) was used (Evorel™), with a starting dose of 12.5 mcg increasing to 25 mcg (patch changed twice weekly) after 4 months. Follow up was every 2 months for a total of 8 months. MEASUREMENTS: Uterine dimensions using ultrasound, oestradiol concentrations and breast development assessed by both Tanner staging and 3D photographic imaging. RESULTS: After 8 months of treatment, the changes in oestradiol concentrations (0-174 pmol), uterine volume growth (4.4-16.4 ml) and breast volume (1.76-140.1 ml) varied greatly between individuals. Of uterine parameters, transverse uterine diameter was most closely associated with serum oestradiol levels at 8 months (beta standardised coefficient = 0.80, p = .001). Change in breast volume was associated with age of treatment initiation (beta standardised coefficient 0.55 p = .04). CONCLUSIONS: We demonstrate variation in response to exogenous oestrogen, emphasising the necessity for individualised dose titration. In the absence of sensitive oestradiol assays, uterine transverse measurements may be used as a surrogate marker of oestrogen sensitivity to guide early dose adjustment. 3D breast imaging may provide a quantitative assessment of breast development to complement Tanner breast staging.

Effect of police enforcement and extreme social inequalities on violence and mental health among women who sell sex: findings from a cohort study in London, UK.

OBJECTIVES: To examine legal and social determinants of violence, anxiety/depression among sex workers. METHODS: A participatory prospective cohort study among women (inclusive of transgender) ≥18 years, selling sex in the last 3 months in London between 2018 and 2019. We used logistic generalised estimating equation models to measure associations between structural factors on recent (6 months) violence from clients or others (local residents, strangers), depression/anxiety (Patient Health Questionnaire-4). RESULTS: 197 sex workers were recruited (96% cisgender-women; 46% street-based; 54% off-street) and 60% completed a follow-up questionnaire. Street-based sex workers experienced greater inequalities compared with off-street in relation to recent violence from clients (73% vs 36%); police (42% vs 7%); intimate partner violence (IPV) (56% vs 18%) and others (67% vs 17%), as well as homelessness (65% vs 7%) and recent law enforcement (87% vs 9%). Prevalence of any STI was 17.5% (17/97). For street-based sex workers, recent arrest was associated with violence from others (adjusted OR (aOR) 2.77; 95% CI 1.11 to 6.94) and displacement by police was associated with client violence (aOR 4.35; 95% CI 1.36 to 13.90). Financial difficulties were also associated with client violence (aOR 4.66; 95% CI 1.64 to 13.24). Disability (aOR 3.85; 95% CI 1.49 to 9.95) and client violence (aOR 2.55; 95% CI 1.10 to 5.91) were associated with anxiety/depression. For off-street sex workers, financial difficulties (aOR 3.66; 95% CI 1.64 to 8.18), unstable residency (aOR 3.19; 95% CI 1.36 to 7.49), IPV (aOR 3.77; 95% CI 1.30 to 11.00) and alcohol/drug use were associated with client violence (aOR 3.16; 95% CI 1.26 to 7.92), while always screening and refusing clients was protective (aOR 0.36; 95% CI 0.15 to 0.87). Disability (aOR 5.83; 95% CI 2.34 to 14.51), unmet mental health needs (aOR 3.08; 95% CI 1.15 to 8.23) and past eviction (aOR 3.99; 95% CI 1.23 to 12.92) were associated with anxiety/depression. CONCLUSIONS: Violence, anxiety/depression are linked to poverty, unstable housing and police enforcement. We need to modify laws to allow sex workers to work safely and increase availability of housing and mental health services.

'Botched labiaplasty': a content analysis of online advertising for revision labiaplasty.

A new development in female genital cosmetic surgery (FGCS) is the promotion of revision surgery for 'botched labiaplasty'. This content analysis study reviews the quality of information offered on websites specifically advertising revision labiaplasty. Twelve websites were identified through online searches and were examined for the quality of their clinical information. All sites defined botched labiaplasty as unsatisfactory appearance after labiaplasty. Four gave no further details and five listed asymmetry, irregular labial edges or removal of too much or too little tissue. Four websites described primary botched labiaplasty as 'mutilation'. Inadequacy of the primary surgeon was cited as the cause of botched labiaplasty in 11/12. Only two websites mentioned risks of surgery. Good outcomes were not defined and no website provided outcome data although guaranteed satisfaction was implied in two websites. This study highlights the existence and promotion of services for botched labiaplasty using non-specific and emotive descriptions. These findings suggest that unsatisfactory results from consumers' perspectives are far from uncommon. The same women whose expectations have not been met by primary surgery are now being targeted for repeat surgery with online advertising capitalising on their unchanged motivations.Impact StatementWhat is already known on this subject? Female genital cosmetic surgery (FGCS) is mainly advertised online with labiaplasty as the most commonly performed procedure. A market for labiaplasty revision to correct 'botched' primary procedures is developing. Academic literature and advertising materials are inconsistent when defining indications and determinants of success for labiaplasty or revision.What the results of this study add? A content analysis of websites specifically advertising revision labiaplasty describes the emotive and nonspecific terms used online to promote revision labiaplasty.What the implications are of these findings for clinical practice and/or further research? The existence of services for botched labiaplasty suggests dissatisfaction is common. Women whose expectations have not been met by primary surgery are targeted for repeat surgery through online advertising capitalising on their potentially unchanged motivations. This study demonstrates the need for clearer outcome data for labiaplasty and highlights the need for better advertising standards for FGCS promotion.

Establishing reproductive potential and advances in fertility preservation techniques for XY individuals with differences in sex development.

BACKGROUND: Discordance between gonadal type and gender identity has often led to an assumption of infertility in patients with differences in sex development (DSD). However, there is now greater recognition of fertility being an important issue for this group of patients. Currently, gonadal tissue that may have fertility potential is not being stored for individuals with DSD and, where gonadectomy forms part of management, is often discarded. The area of fertility preservation has been predominantly driven by oncofertility which is a field dedicated to preserving the fertility of patients undergoing gonadotoxic cancer treatment. The use of fertility preservation techniques could be expanded to include individuals with DSD where functioning gonads are present. METHODS: This is a systematic literature review evaluating original research articles and relevant reviews between 1974 and 2018 addressing DSD and fertility, in vitro maturation of sperm, and histological/ultrastructural assessment of gonadal tissue in complete and partial androgen insensitivity syndrome, 17ß-hydroxysteroid dehydrogenase type 3 and 5α-reductase deficiency. CONCLUSION: Successful clinical outcomes of ovarian tissue cryopreservation are paving the way for similar research being conducted using testicular tissue and sperm. There have been promising results from both animal and human studies leading to cryopreservation of testicular tissue now being offered to boys prior to cancer treatment. Although data are limited, there is evidence to suggest the presence of reproductive potential in the gonads of some individuals with DSD. Larger, more detailed studies are required, but if these continue to be encouraging, individuals with DSD should be given the same information, opportunities and access to fertility preservation as other patient groups.

An exploration of attitudes towards female genital mutilation (FGM) in men and women accessing FGM clinical services in London: a pilot study.

This pilot study researched the attitudes towards and the knowledge of female genital mutilation (FGM) in adult women with FGM and their partners. The participant population consisted of English-speaking women and men over 18 years old attending specialist FGM clinics in two London hospitals. The participants completed a questionnaire on the attitudes and the knowledge of FGM, which were adapted with permission from the United Nations Children's Fund and the United States Agency for International Development household surveys. 54 participants (51 women, 3 men) took part in the surveys. 89% of participants thought that FGM should be stopped (95%CI: 0.81-0.97) and 72% said they knew FGM is illegal in the United Kingdom (UK). 15% reported that FGM caused no danger, or were unaware of any danger to women's health. This study demonstrates the opposition to FGM by participants, but some lack of knowledge regarding the legal and health implications. The exploration of attitudes in diaspora community groups is often cited as key to safeguarding girls from FGM. This is one of the first UK studies of individuals from FGM-practising communities, and we recommend use of the study questionnaires for a multicentre, cross-community study. Impact statement What is already known about this subject? Women and children are affected by female genital mutilation (FGM) globally and in the United Kingdom (UK). The majority of knowledge on practices and the attitudes towards FGM comes from UNICEF and USAID research in Africa and there is scant data on FGM practices in diaspora communities in the UK. What do the results of this study add? This study provides an appropriate questionnaire and protocol for use in community-based national research to improve healthcare for women by collecting up-to-date data on the attitudes towards FGM among the members of FGM-practising communities in the UK. What are the implications of these findings for clinical practice and further research? The implications of the results of this study are that health professionals need to understand that patients do not always know the law on FGM, even after a consultation. Health and social care professionals are placed in a unique position to work with community members to educate men and women to end FGM.

Bone mineral density in complete androgen insensitivity syndrome and the timing of gonadectomy.

OBJECTIVE: Low bone mineral density (BMD) has been reported in complete androgen insensitivity syndrome (CAIS), but the impact of timing of gonadectomy is not known. We aimed to assess the relationship between age of gonadectomy and BMD in women with CAIS. DESIGN: Retrospective analysis of pre- and post-gonadectomy parameters in women with CAIS attending an adult Disorders of Sex Development (DSD) clinic in a tertiary centre. PATIENTS: One hundred and thirteen women with CAIS. MEASUREMENTS: Dual-energy X-ray absorptiometry (DXA) before and after gonadectomy; and pre-gonadectomy hormone profile. RESULTS: Mean BMD was reduced (95% confidence interval); T-score -1.34 (-1.55 to -1.13; P<.001) at the lumbar spine and -0.3 (-0.49 to -0.12; P=.001) at the hip. There was no relationship between age of gonadectomy and BMD. Thirty-two subjects had BMD measured before or within 2 years of gonadectomy, and mean BMD was reduced (95% CI) at the lumbar spine; T-score: -1.05 (-1.54 to -0.57; P<.001), but was normal at the hip; T-score -0.04 (-0.35 to 0.28; P=.8). There was no relationship between BMD and history of hernia, testosterone, oestradiol or follicle stimulating hormone levels. Twelve subjects had DXA both before and after gonadectomy, and after 4.3 (1.7-12.8) years, there was no change in BMD. CONCLUSIONS: We found reduced BMD at the spine and hip in subjects with CAIS. We found no relationship between age of gonadectomy and BMD, and we also found no drop in BMD in subjects followed up after gonadectomy.

Setting up a clinic to assess children and young people for female genital mutilation.

It is now mandatory for health, social care professionals and teachers to report to the police all under-18s where female genital mutilation (FGM) has been disclosed by the child or where physical signs of FGM are seen. Such referrals are likely to result in a request for medical examination. New multiagency statutory guidance sets out instructions for physical examination but provides no details how services should be set-up. This review gives practical guidance learnt from the first year of the UK's only dedicated children's FGM service.

Adolescent and adult outcomes in women following childhood vaginal reconstruction for cloacal anomaly.

PURPOSE: We examined outcomes in female adolescents and women who underwent vaginoplasty in childhood during genitourinary reconstruction for cloacal anomalies. MATERIALS AND METHODS: We retrospectively reviewed the medical notes on girls and women attending an adult specialist center for genitourinary anomalies. Data were collected on vaginal reconstruction, menstruation, sexual and reproductive function, and urological and gastroenterological outcomes. RESULTS: We identified 19 patients with a mean age of 22 years (range 13 to 35), of whom 16 (84%) underwent vaginoplasty in the first year of life. Nine of these 16 patients (56%) had required 1 (7) or 2 (2) further vaginal reconstructions to facilitate menstruation or sexual activity. The remaining 7 patients (44%) required no further vaginal reconstruction. Nine of the 19 patients (47%) had associated müllerian anomalies, obstructed menstruation developed in 5 (26%) and 1 required hemihysterectomy. Eight patients were sexually active, of whom 1 experienced difficult penetration. Three patients attempted to conceive, including 1 with a complex preterm delivery and 2 undergoing fertility treatment. Of the patients 74% underwent further reconstruction of the renal tract and 36% had an enteric stoma. CONCLUSIONS: This study confirms the complexity of vaginal reconstruction in this group with a notable vaginoplasty revision rate. Müllerian anomalies were identified in almost half of the patients, a higher incidence than previously reported, and in a quarter obstructed menstruation developed in puberty. A specialist team with gynecologic input should treat patients with cloacal anomalies. Outcome data are sparse. There remains a need for well planned, prospective cohort studies that include assessments of psychological, sexual and reproductive outcomes.

Developing and testing accelerated partner therapy for partner notification for people with genital Chlamydia trachomatis diagnosed in primary care: a pilot randomised controlled trial.

BACKGROUND: Accelerated partner therapy (APT) is a promising partner notification (PN) intervention in specialist sexual health clinic attenders. To address its applicability in primary care, we undertook a pilot randomised controlled trial (RCT) of two APT models in community settings. METHODS: Three-arm pilot RCT of two adjunct APT interventions: APTHotline (telephone assessment of partner(s) plus standard PN) and APTPharmacy (community pharmacist assessment of partner(s) plus routine PN), versus standard PN alone (patient referral). Index patients were women diagnosed with genital chlamydia in 12 general practices and three community contraception and sexual health (CASH) services in London and south coast of England, randomised between 1 September 2011 and 31 July 2013. RESULTS: 199 women described 339 male partners, of whom 313 were reported by the index as contactable. The proportions of contactable partners considered treated within 6 weeks of index diagnosis were APTHotline 39/111 (35%), APTPharmacy 46/100 (46%), standard patient referral 46/102 (45%). Among treated partners, 8/39 (21%) in APTHotline arm were treated via hotline and 14/46 (30%) in APTPharmacy arm were treated via pharmacy. CONCLUSIONS: The two novel primary care APT models were acceptable, feasible, compliant with regulations and capable of achieving acceptable outcomes within a pilot RCT but intervention uptake was low. Although addition of these interventions to standard PN did not result in a difference between arms, overall PN uptake was higher than previously reported in similar settings, probably as a result of introducing a formal evaluation. Recruitment to an individually randomised trial proved challenging and full evaluation will likely require service-level randomisation. TRIAL REGISTRATION NUMBER: Registered UK Clinical Research Network Study Portfolio id number 10123.

Sexual function and health-related quality of life in women with classic bladder exstrophy.

OBJECTIVE: To investigate sexual function and quality of life in adolescent and adult women with classic bladder exstrophy (BE). MATERIALS AND METHODS: A two-part observational cross-sectional study with a questionnaire arm and a retrospective case review arm was performed. The study was undertaken in a centre providing a tertiary referral gynaecology and urology service. Outcomes were sexual function and quality-of-life scores. RESULTS: A total of 44 patients with BE were identified from departmental databases and included in the study, of whom 28 (64%) completed postal questionnaires. Sexual function scores and quality-of-life visual analogue scales were significantly poorer compared with normative data. CONCLUSIONS: Bladder exstrophy has a detrimental psychological impact on women. In future, methodical multidisciplinary paediatric follow-up research will help to identify predictors of better and worse adolescent and adult outcomes. Development and evaluation of cost-effective psychological interventions to target specific problems is also warranted.

The long-term management and outcomes of cloacal anomalies.

Cloacal anomalies occur when failure of the urogenital septum to separate the cloacal membrane results in the urethra, vagina, rectum and anus opening into a single common channel. The reported incidence is 1:50,000 live births. Short-term paediatric outcomes of surgery are well reported and survival into adulthood is now usual, but long-term outcome data are less comprehensive. Chronic renal failure is reported to occur in 50 % of patients with cloacal anomalies, and 26-72 % (dependant on the length of the common channel) of patients experience urinary incontinence in adult life. Defaecation is normal in 53 % of patients, with some managed by methods other than surgery, including medication, washouts, stoma and antegrade continent enema. Gynaecological anomalies are common and can necessitate reconstructive surgery at adolescence for menstrual obstruction. No data are currently available on sexual function and little on the quality of life. Pregnancy is extremely rare and highly risky. Patient care should be provided by a multidisciplinary team with experience in managing these and other related complex congenital malformations. However, there is an urgent need for a well-planned, collaborative multicentre prospective study on the urological, gastrointestinal and gynaecological aspects of this rare group of complex conditions.

A gynaecologic perspective on cloacal malformations.

PURPOSE OF REVIEW: Advances in surgical reconstruction of cloacal malformations have led to better functional outcomes and quality of life. As a result, adolescents and women born with these complex malformations will have the same aspirations as their peers including sexual relationships and fertility. RECENT FINDINGS: Currently, there is a paucity of data on gynaecologic outcomes and sexual function, and obstetric data are limited primarily to case reports. Making evidence-based clinical recommendations is difficult for gynaecologic providers. Unique gynaecologic issues can arise in infancy, adolescence or adulthood. Recognition and appropriate management of these complications is imperative to maximize sexual esteem and preserve future fertility. Pregnancy requires adequate prenatal preparation and specialized multidisciplinary care under an experienced obstetrician and urologist. SUMMARY: This review highlights the issues that may be encountered in providing gynaecologic care to patients with cloacal malformations, presents the available literature to provide informative evidence and identifies gaps in knowledge in order to suggest potential future research opportunities.

Contribution of internal noise and calculation efficiency to face discrimination deficits in older adults.

Classification images (CIs) measured in a face discrimination task differ significantly between older and younger observers. These age differences are consistent with the hypothesis that older adults sample diagnostic face information less efficiently, or have higher levels of internal noise, compared to younger adults. The current experiments assessed the relative contributions of efficiency and internal noise to age differences in face discrimination using the external noise masking and double-pass response consistency paradigms. Experiment 1 measured discrimination thresholds for faces embedded in several levels of static white noise, and the resulting threshold-vs.-noise curves were used to estimate calculation efficiency and equivalent input noise: older observers had lower efficiency and higher equivalent input noise than younger observers. Experiment 2 presented observers with two identical sequences of faces embedded in static white noise to measure the association between response accuracy and response consistency and estimate the internal:external (i/e) noise ratio for each observer. We found that i/e noise ratios did not differ significantly between groups. These results suggest that age differences in face discrimination are due to differences in calculation efficiency and additive internal noise, but not to age differences in multiplicative internal noise.

Mayer-Rokitansky-Kuster-Hauser syndrome: diagnosis with MR imaging.

PURPOSE: To evaluate the diverse magnetic resonance (MR) imaging findings of the pelvis in women with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. MATERIALS AND METHODS: This retrospective review had institutional review board approval with waiver of informed consent. Between 2001 and 2011, 215 female patients with MRKH syndrome attended clinics, and 66 underwent pelvic MR imaging (age range, 14-40 years; median age, 19 years). One reviewer reviewed MR images for presence, site, volumes, and differentiation into layers (myometrium, junctional zone, and endometrium) of uterine remnants. Ovarian volumes and positions were assessed. Vaginal length was measured. RESULTS: Rudimentary uteri were found in 61 patients (92%); 54 were bilateral, and seven were unilateral. All uterine buds were located laterally in the pelvis and had a constant caudal relationship with their paired ovary. Mean uterine volume was 6.4 mL (range, 0.4-80.2 mL), and 18 uteri had a volume greater than 10 mL. Twenty-four uterine buds (21%) showed differentiation into more than one layer. Two uteri contained intraluminal blood, and two showed signs of adenomyosis, indicating functioning endometrial tissue; these patients had cyclical pain. Bilateral ovaries were present in 54 patients; ovaries were ectopic in 27 patients. Twenty-two patients had no discernible vagina (dimple or less). Of the 44 patients with a vagina, the mean length was 2.0 cm (range, 1.0-6.5 cm). CONCLUSION: Rudimentary uteri are common in patients with MRKH syndrome. They can be relatively large and have functioning endometrium, which can be associated with pain. Uteri have a constant caudal relationship to ovaries. Ovaries are commonly ectopic, and this must be recognized in patients undergoing fertility treatment. Online supplemental material is available for this article.

Evaluation of retained testes in adolescent girls and women with complete androgen insensitivity syndrome.

PURPOSE: To evaluate the magnetic resonance (MR) imaging appearance of the testes in women with complete androgen insensitivity syndrome (CAIS), including any benign or malignant changes. MATERIALS AND METHODS: This was a retrospective review of the testicular MR images and histologic reports from 25 patients with CAIS who chose to retain their testes beyond age 16 years and who were imaged between January 2004 and December 2010. Ethical approval was obtained, and informed consent was obtained from each subject to review the medical records, images, and histologic slides and reports. Imaging and histologic findings were compared. RESULTS: Twelve patients (mean age, 24 years; age range, 18-39 years) retained their testes and 13 (mean age, 22 years; age range, 17-37 years) eventually underwent gonadectomy. Review of the MR images showed that testicular parenchyma was heterogeneous in 30 of 46 testes (65%). The most common changes on MR images included simple-looking paratesticular cysts (34 of 46 testes, 74%) and low-signal-intensity, well-defined Sertoli cell adenomas (26 of 46 testes, 56%). Correlation of the histologic and MR imaging findings showed that MR imaging could correctly depict the presence or absence of Sertoli cell adenomas in 19 of 23 testes (83%). Paratesticular cysts were correctly detected in 22 of 23 testes (96%). Microscopic examination showed that the testes were composed of atrophic seminiferous tubules, whereas germ cells were found in 13 of 26 testes (50%). All paratesticular cysts were confirmed to be benign; however, a focus of intratubular germ cell neoplasia was found in a Sertoli cell adenoma. Premalignant foci were detected in three patients, two with intratubular germ cell neoplasia and one with sex cord tumor with annular tubules. No invasive cancers were found. CONCLUSION: MR imaging is accurate in the detection of testicular changes, including paratesticular cysts and Sertoli cell adenomas. Although these changes are usually benign, Sertoli adenomas can sometimes harbor premalignant lesions. MR imaging cannot depict premalignant changes; therefore, the standard of care for patients with CAIS should remain gonadectomy after puberty.

Lower urinary tract symptoms in women with vaginal agenesis.

INTRODUCTION AND HYPOTHESIS: Congenital vaginal aplasia is a condition with devastating implications for fertility and sexuality. However, little is known on whether urinary symptomatology is more common prior and following vaginal lengthening procedures in these women. METHODS: We performed a prospective observational study of 19 women with vaginal agenesis before and after vaginal dilation treatment or a laparoscopic Vecchietti procedure. All women completed the ICIQ FLUTS questionnaire before and after treatment in order to assess incidence and changes in urinary symptomatology. RESULTS: Urinary symptoms were present in 53% of women prior to treatment. The majority of bladder symptoms were not significantly altered by treatment, except for incomplete bladder emptying. Women following the Vecchietti procedure had increased urinary hesitancy and a poorer steam compared with those completing dilation treatment. The longer the vaginal length, the greater the urinary frequency. CONCLUSION: Women with vaginal agenesis had significant levels of urinary symptoms and to our knowledge this has not been reported before. The majority of symptoms were not altered by treatment.

Complex gynaecological and urological problems in adolescents: challenges and transition.

The diagnosis and early management of genitourinary disorders in children is challenging for the child, parents and carers. There have been enormous developments in paediatric care medically and surgically and as a result many patients with complex anomalies reach adult life and have a normal desire for a good quality of life. Adolescence changes the challenges faced as anatomical growth may alter the surgical outcomes both functionally and cosmetically. Emotional and psychological development also creates important challenges that all those involved have to face. These patients need expert care and advice throughout adolescence and into adult life. This should be delivered by a multidisciplinary team that includes urology, gynaecology, nephrology, endocrinology and psychology. This creates a safe clinical and supportive environment for patients and their families.

Adult care of children from pediatric urology: part 2.

PURPOSE: We describe the outcomes of undescended testes and sex development disorders in adolescence and young adulthood. We reviewed the requirements for the long-term care of children born with these and other major congenital anomalies of the genitourinary system. MATERIALS AND METHODS: The current English language literature was retrieved with a PubMed® search for articles on these subjects. Only articles covering outcomes at ages past puberty were included in analysis. The material was supplemented from the database of the clinic for adults with sex development disorders at University College London Hospitals. RESULTS: An undescended testis has impaired spermatogenesis. In men in whom a unilateral undescended testis was corrected before puberty the incidence of paternity is normal at around 90% of those who attempt it. The equivalent rate for those with bilateral undescended testes is about 65%. If surgery for bilateral undescended testes is delayed until after puberty, fertility is unlikely. The risk of testicular neoplasms is overestimated and the relative risk is between 2.5 and 8. Children born with a sex development disorder receive multidisciplinary treatment throughout childhood and require the same care as adults. Males who are under virilized likely have a micropenis (greater than 2 SD below the mean stretched length) but they may have normal sexual function. Fertility depends on the underlying condition. Virilized females, who most commonly have congenital adrenal hyperplasia, currently present to adult clinics with an inadequate vagina after infantile surgery. Reconstruction is required to allow intercourse. CONCLUSIONS: The care of adults born with abnormalities of the genitalia is complex. Early management may define upbringing in childhood but requirements for sexuality and fertility in adult life are different. Multidisciplinary care is essential and a case can be made to establish a subspecialty of urology to coordinate it.

Timing of gonadectomy in adult women with complete androgen insensitivity syndrome (CAIS): patient preferences and clinical evidence.

OBJECTIVE: Adult women with complete androgen insensitivity syndrome (CAIS) are increasingly likely to defer or decline gonadectomy despite counselling about malignancy risk. The objectives of this study were to review the evidence on the risk of gonadal malignancy in adult women with CAIS and to explore women's reasons for deferring gonadectomy. STUDY DESIGN: A case series and literature review. PATIENTS: Sixteen women with CAIS over the age of 18 years who have elected to defer gonadectomy. RESULTS: Sixty-two relevant papers were identified. Of these, 14 confirmed that tumours had been reported in 98 adults. Taking into account the limitations of combining historic case series, this review estimates a risk of gonadal malignancy of 14% (range 0% and 22%) in adults with CAIS. The most common reasons women offered for deferring gonadectomy included inconvenience of surgery, concern about surgical risk and reluctance to take hormone replacement therapy. CONCLUSIONS: Perceived benefits for retaining gonads in women with CAIS are prompting more women to keep their gonads in situ. An accurate estimate for adult malignancy risk is unavailable, and the risks currently quoted may be falsely reassuring.

Reproductive outcomes in women with classic bladder exstrophy: an observational cross-sectional study.

OBJECTIVE: We sought to examine the reproductive outcomes of 52 women with classical bladder exstrophy. STUDY DESIGN: This was an observational study with cross-sectional and retrospective arms. RESULTS: The average age of the sample was 33 years (range, 17-63). Of those who had tried, 19/38 (66%) had conceived. A total of 57 pregnancies (3 sets of twins) were reported for the 19 patients and resulted in 34/57 live births (56%), 21/57 miscarriages (35%), 1/57 (2%) termination, and 4/57 (7%) stillbirths or neonatal deaths. Four deliveries resulted in major complications including 1 transection of the ureter (4%), 1 fistula formation (4%), and 2 postpartum hemorrhages (8%). There were 2 admissions to intensive care, one for urinary sepsis and another for massive obstetric hemorrhage. CONCLUSION: Fertility is impaired in women with bladder exstrophy. Pregnancy is high risk both for the mother and baby. Delivery should be at a tertiary referral obstetric unit with urology cover. In the majority of cases planned cesarean section is the most appropriate mode of delivery.